| 摘要: |
| 成骨不全是一组以骨骼脆性增加及胶原代谢紊乱为特征的全身性结缔组织疾病，主要由编码Ⅰ型胶原的基因发生突变所致，此病以骨脆性增加、骨关节进行性畸形、蓝巩膜、牙本质发育不全及听力下降为常见表现。目前治疗成骨不全的最理想的药物是双膦酸盐，本文复习国内外相关文献，就其治疗的最适剂量与时间、最佳给药途径、最佳药物选择以及药物与成骨不全类型和患者年龄的最适匹配等问题的研究进展作一综述。 |
| 关键词: 双膦酸盐类 成骨不全 药物疗法 治疗结果 |
| DOI：10.3724/SP.J.1008.2014.00200 |
| 投稿时间：2013-07-07修订日期：2013-09-28 |
| 基金项目:国家自然科学基金（81201366），第二军医大学青年启动基金（2011QN15）. |
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| Bisphosphonate in treatment of osteogenesis imperfecta：an update |
| SHI Chang-gui,ZHANG Ying,YUAN Wen* |
(Department of Orthopaedics, Changzheng Hospital, Second Military Medical University, Shanghai 200003, China
*Corresponding author.) |
| Abstract: |
| Osteogenesis imperfecta is a group of systemic connective tissue diseases characterized by increase in bone fragility and collagen metabolic disorder. The condition is mainly caused by mutations in the genes encoding type Ⅰ collagen. The clinical features of the diseases include bone fragility, progressive bone and joint deformity, blue sclera, dentinogenesis imperfecta and hearing loss. Currently, the most promising drug for treatment of the disease is bisphosphonate. This article reviews the following issues: optimal dose and duration of treatment, best route for administration, best drug choice, and the optimal match of drugs with the osteogenesis imperfecta type and age of patients. |
| Key words: bisphosphonates osteogenesis imperfecta drug therapy treatment outcome |
